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October 1929

Untersuchungen ueber Porphyrie mit besonderer Berücksichtigung der Porphyria congenita.

Arch Intern Med (Chic). 1929;44(4):623. doi:10.1001/archinte.1929.00140040161017

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The book is based on a study of a case of congenital porphyry in a man who died at the age of 32. His condition had been investigated clinically and biochemically in great detail and had previously been reported by Fischer, Gunther and Weiss. Borst reports the gross and microscopic observations of the autopsy. Grossly, there was porphyrin discoloration or "hemosiderosis" of the skin and the entire skeleton, spleen, liver, lymph nodes, pancreas and kidneys, and histologically of practically all of the tissues of the body. Microhistochemical and physical analyses of the pigments in the various organs and tissues were made by Fischer. Königsdorffer studied the fate of porphyrins administered experimentally to animals. They came to the conclusion that porphyry is a constitutional anomaly. There is not an incomplete synthesis of hemoglobin, myoglobulin and histohematin or cytochrome as has been claimed. They believe that in congenital porphyry there is an

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